SlideShare uma empresa Scribd logo
1 de 62
 INTRODUCTION
 EPIDEMIOLOGY
 PATHOGENESIS
 PATHOLOGY
 CLINICAL FEATURES
 LABORATORY FINDINGS
 DIAGNOSIS
 TREATMENT
 PREVENTION
 REFERENCES
Acute rheumatic fever (ARF) is a multisystem disease
resulting from an autoimmune reaction to infection
with group A streptococci.
 ARF and RHD are diseases of poverty.
 It is a postsuppurative streptococcal pharyngitis
cascade, leading variably to arthritis , chorea , dermal
manifestations and most importantly, carditis.
 The incidence of RF has declined dramatically in
industrialized nations , but remains common in
developing nations.
Accounts for less than 1% of cardiac manifestations in
industrialized countries.
RHD is the most common cause of heart disease in
children in developing countries and is a major cause of
mortality and morbidity in adults as well.
 Estimated in 2005 that approximately 15.6 million people
had RF or RHD.
 Age group:5 to 14 years.
 Initial episodes become less common in older
adolescents and young adults and are rare in persons
aged >30 years.
 Recurrent episodes of ARF remain relatively common
in adolescents and young adults.
 No clear gender , race predisposition
 But post-pubertal chorea and the development of
mitral stenosis is more common in females.
 Classic triad of agent ,host and environmental all play a
major role.
AGENT FACTORS:
 infection of the upper respiratory tract with group A
beta hemolytic streptococci.
 more than 100 subtypes defined by M protein
surface molecules.
 strains that cause rheumatic fever are -M types
1,3,5,6,18 and 24.
Diagrammatic structure of the group A beta
hemolytic streptococcus
Capsule
Cell wall
Protein antigens
Group carbohydrate
Peptidoglycan
Cyto.membrane
Cytoplasm
HOST FACTORS:
Susceptibility to ARF is an inherited characteristic.
 HLA class II alleles.
 High levels of circulating mannose-binding lectin
 Polymorphisms of transforming growth factor β1
gene and immunoglobulin genes.
 High-level expression of a particular alloantigen
present on B cells, D8-17, has been found in patients
with a history of ARF in many populations.
 Prior history of rheumatic fever is also an important
risk factor.
ENVIRONMENTAL FACTORS:
 overcrowded housing,
 poor personal and community hygiene,
 poor access to medical services.
THE IMMUNE RESPONSE:
 An autoimmune reaction results, which leads to
damage to human tissues.
 Cross-reactivity between epitopes on the organism
and the host.
 Epitopes present in the cell wall, cell membrane, and
the A, B, and C repeat regions of the streptococcal M
protein are immunologically similar to molecules in
human:
 myosin, tropomyosin(myocardium),
 keratin (skin)
 actin,
 laminin(valves),
 vimentin(synovia),
 N-acetylglucosamine.
 lysogangliosides(subthalamic and caduate nuclei in
the brain).
 This molecular mimicry is the basis for the
autoimmune response that leads to ARF.
 Laminin, α-helical coiled protein like myosin and
M protein, found in cardiac endothelium recognized
by anti-myosin, anti-M protein T cells.
 Antibodies to cardiac valve tissue cross-react with
the N-acetylglucosamine of group A streptococcal
carbohydrate.
 These antibodies may be responsible for valvular
damage.
Pathogenetic pathway for acute rheumatic fever and rheumatic heart
disease.
 Verrucous vegetations on the valve leaflets along the
lines of closure, with extensive inflammation and
edema.
EXUDATIVE PHASE:
 first few weeks after the onset of RF
 fibrinoid degeneration of collagen
 inflammation in left ventricular endocardium.
PROLIFERATIVE PHASE:
 from 1 to 6 months after the onset of RF.
 ASCHOFF BODIES ,granulomatous lesions
pathognomonic for rheumatic carditis .
 found in valve tissue as well as endocardium,
myocardium and pericardium.
Microscopic appearance of Aschoff body in a patient with acute
rheumatic carditis.
Rheumatic arthritis is manifest by
 edema,
lymphocytic and polymorphonuclear infiltration,
 fibrinoid lesions that resolve.
In patients with chorea , inflammatory changes have
been noted in the cerebral cortex ,cerebellum and
basal ganglia.
 Latent period of ~3 weeks (1–5 weeks) between the
precipitating group A streptococcal infection and the
appearance
of the clinical features of ARF.
Exceptions are chorea and indolent carditis,which may
follow prolonged latent periods lasting up to 6 months.
Clinical manifestations:
 Fever
 Polyarthritis
 Carditis
 Sydenham’s chorea
 Erythema marginatum.
 Subcutaneous nodules.
Most common is fever ,polyarthritis followed by
carditis.
CARDITIS:
Occurs in 50-60% of patients with ARF.
 The endocardium, pericardium, or myocardium
may be affected.
 Valvular damage is the hallmark of rheumatic
carditis.
 almost always affects the mitral valve causing MR.
 sometimes together with aortic valve.
 isolated aortic valve involvement with AR is rare.
 acute and chronic myocardial dysfunction.
 acute ,although not chronic pericardial disease.
 neither pericarditis nor myocarditis can be expected to
occur in the absence of valvulitis.
 Rheumatic tricuspid disease is uncommon ,and
pulmonic valve disease is rare.
 Severe heart failure in acute RF is secondary to altered
myocardial mechanics caused by MR rather than secondary
to myocarditis.
• Severity of left ventricular dysfunction appears to
correlate with the extent of valvulitis rather than with any
myocardial injury.
Over ensuing years, usually as a result of recurrent
episodes, leaflet thickening, scarring, calcification,
and valvular stenosis may develop.
Pericarditis most commonly causes a friction rub or a
small effusion on echocardiography and may
occasionally cause pleuritic central chest pain.

Myocardial inflammation may affect electrical
conduction pathways ,leading to P-R interval
prolongation (first-degree AV block or rarely higher-
level block) which resolves over a few days to weeks.
 JOINT INVOLVEMENT:
 Polyarthrits is most common manifestation of RF ,
occuring in 60 to 75% of patients.
To qualify as a major manifestation, joint involvement
must be arthritic, i.e., objective evidence of
inflammation ,with hot, swollen, red and/or tender joints
and involvement of more than one joint (i.e., polyarthritis).
 typically migratory ,moving from one joint to another
over a period of hours.
 almost always affects the large joints— the knees,
ankles, hips, and elbows
 asymmetric.
 Arthralgia without objective joint inflammation -
minor criteria.
 Inflammation in individual joints lasts 1 to 2 weeks.
 Polyarthritis as a whole resolves in a month or less.
 Chronic sequelae and disability do not occur ,with
the rare exception of Jaccoud arthropathy.
• Arthritic phase frequently overlaps with the onset of
carditis and the two manifestations appear to be
inversely proportional.
 Joint manifestations are highly responsive to
salicylates and other nonsteroidal anti-inflammatory
drugs.
CHOREA
 Sydenham’s chorea occurs after a prolonged latent
period of upto 6 months.
 Reported incidence has a wide range between 5 and
35%.
 Found mainly in females.
 Affects particularly the head and upper limb.
 Manifests as involuntary , irregular movements,
fibrillatory muscle movements of tongue.
 Characteristic spooning with exterernal rotation of the
hands.
 Abolition with sleep.
 There is a substantial risk of subsequent RHD in these
patients.
 Eventually resolves completely, usually within 6 weeks.
 Other psychological and neurological manifestations of
RF:
 Short term and long term emotional liability.
 Obsessive –compulsive disorder.
 Seizures
 Chronic migraine
SKIN MANIFESTATIONS:
Occur in <5% of the cases.
Erythema marginatum:
 Classic rash of ARF
 Begins as pink macules that clear centrally, leaving a
serpiginous, spreading edge.
 The rash is evanescent, appearing and disappearing
before the examiner’s eyes.
 It occurs usually on the trunk, sometimes on the
limbs, but almost never on the face.
 Typically occur in conjunction with carditis.
 May last for months or years.
 Not specific for RF and occurs with sepsis and drug
reaction.
Subcutaneous nodules:
 Painless, small , mobile lumps beneath the skin
overlying bony prominences, of the hands, feet,
elbows, occiput, and occasionally the vertebrae.
 Typically occur in patients with moderate to
severe rheumatic carditis.
 They are a delayed manifestation, appearing
2–3 weeks after the onset of disease, last for just a
few days up to 3 weeks.
 Not diagnostic of RF and can be seen with
other autoimmune disorders.
SUBCUTANEOUS NODULE
OTHER FEATURES:
 Fever occurs in most cases of ARF.
 Although high-grade fever (≥39°C) is the rule,
lower grade temperature elevations are not
uncommon.
Polyarthritis

Infectious
Staphylococcus,
gonococcus
Endocarditis,
Lyme disease,
Mycobacterial,fungal,Viral
 Reactive
Poststreptococcal,
Enteric infection
Reiter syndrome,
Inflammatory bowel
Connective tissue disease
Rheumatoid arthritis
Systemic lupus
Systemic vasculitis
 Miscellaneous :
Gout, Leukemia, lymphoma , Sarcoidosis, Cancer ,
Familial Mediterranean fever , Henoch-Schönlein
purpura , Mucocutaneous disorders , “Growth pains” in
children, Serum sickness .
Carditis
 Murmur
Physiological murmur
Mitral valve prolapse
Bicuspid aortic valve
Anemia
Straight back syndrome
 Congenital heart disease
Ventricular septal defect
Subvalvular aortic stenosis
Primum atrial septal defect
 Viral myocarditis
 Endocarditis
 Pericarditis
Chorea
 Familial chorea—Huntington
 Hormone-induced
Oral contraceptives, pregnancy
 Drug-induced
Anticonvulsants , antidepressant,
metoclopramide.
 Connective tissue
Systemic lupus, Periarteritis
 Lyme disease , Wilson disease , Atypical
seizures , Hyperthyroidism,
Hypoparathyroidism , Tourette syndrome,
PANDAs.
Elevated ESR and CRP.
 Mild elevation of the peripheral leucocyte count.
 Evidence of a Preceding Group A Streptococcal
Infection:
 Positive throat swab culture
 Rapid streptococcal antigen test-generally
specific but sensitivity is low
 Rising streptococcal antibody titres which
includes:
a. antistreptolysin O.
b. anti-deoxyribonuclease B.
c. antihyaluronidase
d. streptozyme.
Antibody titre testing is more specific although they
are affected by non-GABHS infections.
Time course of antibody level increase:
 within 1 month of onset of streptococcal
pharyngitis.
 plateaus for 3 t0 6 months, followed by a
decline.
 levels elevated from the patient’s baseline
typically last 1 year or less.
 Electrocardiographic findings:
 Sinus arrythmia
 Tachycardia
 Conduction disturbances-first degree AV
block.
 Prolongation of QT interval.
 Rare episodes of torsades de pointes and
sudden death.
 Echocardiography:
 Miral insufficiency is the most common finding
associated subsequently with restricted leaflet motion
 Occasionally aortic insufficiency has been diagnosed
in a minority of patients.
Echocardiography is useful :
 For confirming the findings on auscultation.
 Excluding non-rheumatic causes(physiological
murmurs or congenital heart disease).
 Sequential follow up of valvular insufficiency,
chamber size ,pulmonary hypertension, valve
thickening and left ventricular systolic function.
 Associated post-streptococcal syndromes:
 Post streptococcal reactive arthritis:
(1) small-joint involvement that is often symmetric.
(2) a short latent period following streptococcal
infection (usually <1 week).
(3) occasional causation by non-group A β-hemolytic
streptococcal infection.
(4) slower responsiveness to salicylates.
(5) the absence of other features of ARF, particularly
carditis.
 Pediatric Autoimmune Neuropsychiatric Disorders
Associated with Streptococcal infection (PANDAS) :
Consists of a range of tic disorders and obsessive-
compulsive symptoms associated with group A
streptococcal infections.
 People with PANDAS are said not to be at risk of
carditis, unlike patients with Sydenham’s chorea.
 The diagnoses of PANDAS and PSRA should rarely be
made in populations with a high incidence of ARF
 Based on the WHO modification of the 1992 Revised
Jones criteria.
Major manifestations:
 Carditis
 Polyarthritis
 Chorea
 Erythema marginatum
 Subcutaneous nodules
 Minor manifestations:
 Clinical: fever, polyarthralgia
 Laboratory: elevated erythrocyte sedimentation
rate or leukocyte count
 Electrocardiogram: prolonged P-R interval
Supporting evidence of a preceding streptococcal
infection within the last 45 days
 Elevated or rising anti-streptolysin O or other
streptococcal antibody, or
 A positive throat culture, or
 Rapid antigen test for group A streptococcus, or
 Recent scarlet fever
Primary episode of rheumatic fever:
 Two major or one major and two minor
manifestations plus evidence of preceding group A
streptococcal infection.
Recurrent attack of rheumatic fever in a patient
without established rheumatic heart disease:
 Two major or one major and two minor
manifestations plus evidence of preceding group A
streptococcal infection
Recurrent attack of rheumatic fever in a patient
with established rheumatic heart disease.
 Two minor manifestations plus evidence of
preceding group A streptococcal infection.
Rheumatic chorea and Insidious onset rheumatic
carditis:
 Other major manifestations or evidence of
group
A streptococcal infection not required.
Chronic valve lesions of rheumatic heart disease
(patients presenting for the first time with pure
mitral stenosis or mixed mitral valve disease
and/or aortic valve disease)
 Do not require any other criteria to be
diagnosed
as having rheumatic heart disease.
ANTIBIOTICS:
 All patients with ARF should receive antibiotics
sufficient to treat the precipitating group A
streptococcal infection.
 Penicillin is the drug of choice
 can be given orally , 500 mg twice daily for 10 days or
 as a single dose of 1.2 million units IM benzathine
penicillin G.
 Erythromycin, 250 mg bid, may be used for patients
with penicillin allergy.
 Followed by long term secondary prophylaxis.
 SALICYLATES AND NSAIDS
Used for the treatment of arthritis, arthralgia, and
fever, once the diagnosis is confirmed.
 Aspirin is the drug of choice.
 Initial dose of 80–100 mg/kg per day in children (4–8
g/d in adults) in 4–5 divided doses for the first few
days up to 2 weeks.
 When the acute symptoms are substantially resolved,
the dose can be reduced to 60–70 mg/kg per day for
a further 2–4 weeks.
 GLUCOCORTICOIDS:
 Can be used in cases of severe carditis (causing heart
failure)
may reduce the acute inflammation and result in more
rapid resolution of failure.
 Prednisolone:1–2 mg/kg per day (maximum, 80 mg)
Intravenous methylprednisolone may be used in very
severe carditis.
 Often required for a few days or up to a maximum of
3 weeks.
 MANAGEMENT OF HEART FAILURE
 BED REST:
Recommended only for arthritis and arthralgia
and for patients with heart failure.
 MANAGEMENT OF CHOREA
 Milder cases can usually be managed by providing a
calm environment.
 In severe chorea, carbamazepine or sodium valproate
are preferred to haloperidol.
 Response may not be seen for 1–2 weeks.
 Medication should be continued for 1–2 weeks after
symptoms subside.
INTRAVENOUS IMMUNOGLOBULIN(IVIG):
 Studies have suggested that IVIg may lead to more
rapid resolution of chorea.
 But has shown no benefit on the short- or long-term
outcome of carditis in ARF without chorea.
 IVIg is not recommended except in cases of severe
chorea refractory to other treatments.
 PRIMARY PREVENTION:
I. Elimination of the major risk factors for
streptococcal infection, particularly overcrowded
housing and inadequate hygiene infrastructure.
II. Primary prophylaxis:
 Timely and complete treatment of group A
streptococcal sore throat with antibiotics.
 If commenced within 9 days of sore throat onset,
a course of 10 days of penicillin V (500 mg bid PO in
adults) or
 A single IM injection of 1.2 million units of
benzathine penicillin G should be administered.
SECONDARY PREVENTION:
 The mainstay of controlling ARF and RHD.
 Patients with ARF should receive long term
penicillin prophylaxis to prevent recurrences.
 The best antibiotic for secondary prophylaxis is
benzathine penicillin G:
1.2 million units, or 600,000 units if <30 kg delivered
every 4 weeks or more frequently to persons
considered to be at particularly high risk.
 Oral penicillin V (250 mg) can be given twice-daily
instead.
 less effective than benzathine penicillin G.
Penicillin allergic patients can receive erythromycin
(250 mg) twice daily.
Harrison’s Principles of Internal Medicine,17th
edt.
 Braunwald’s diseases of the heart,8th
edt.
 Oxford text book of medicine -4th
edt.
 Nelson’s text book of Pediatrics.
 Robbins and Cotran ,Pathological Basis of Diseases,8th
edt.
 Cecil text book of Medicine,22nd
edt.
Acute rheumatic fever-definition,pathophysiology,clinical presentation and management.

Mais conteúdo relacionado

Mais procurados

Fever in infants and children
Fever in infants and childrenFever in infants and children
Fever in infants and children
Mohamed Abunada
 
Infective endocarditis
Infective endocarditisInfective endocarditis
Infective endocarditis
Puneet Shukla
 
Chronic stable angina
Chronic stable anginaChronic stable angina
Chronic stable angina
Muhammad Saim
 
Rheumatic heart disease
Rheumatic heart diseaseRheumatic heart disease
Rheumatic heart disease
adolescent4u
 

Mais procurados (20)

Rheumatic Fever and Rheumatic Heart Disease
Rheumatic Fever and Rheumatic Heart DiseaseRheumatic Fever and Rheumatic Heart Disease
Rheumatic Fever and Rheumatic Heart Disease
 
Acute rheumatic fever
Acute rheumatic feverAcute rheumatic fever
Acute rheumatic fever
 
Myocarditis in children
Myocarditis in childrenMyocarditis in children
Myocarditis in children
 
INFECTIVE ENDOCARDITIS IN CHILDREN
INFECTIVE ENDOCARDITIS IN CHILDRENINFECTIVE ENDOCARDITIS IN CHILDREN
INFECTIVE ENDOCARDITIS IN CHILDREN
 
Infective endocarditis
Infective endocarditisInfective endocarditis
Infective endocarditis
 
Rheumatic heart disease
Rheumatic heart diseaseRheumatic heart disease
Rheumatic heart disease
 
Acute rheumatic fever
Acute rheumatic feverAcute rheumatic fever
Acute rheumatic fever
 
Fever in infants and children
Fever in infants and childrenFever in infants and children
Fever in infants and children
 
Infective endocarditis
Infective endocarditisInfective endocarditis
Infective endocarditis
 
secondary hypertension
secondary hypertensionsecondary hypertension
secondary hypertension
 
Pulmonary embolism
Pulmonary embolismPulmonary embolism
Pulmonary embolism
 
Goodpasture syndrome
Goodpasture syndromeGoodpasture syndrome
Goodpasture syndrome
 
Rheumatic Fever and Rheumatic Heart Disease
Rheumatic Fever and Rheumatic Heart DiseaseRheumatic Fever and Rheumatic Heart Disease
Rheumatic Fever and Rheumatic Heart Disease
 
Chronic stable angina
Chronic stable anginaChronic stable angina
Chronic stable angina
 
Heart failure
Heart failureHeart failure
Heart failure
 
Rheumatic heart disease
Rheumatic heart diseaseRheumatic heart disease
Rheumatic heart disease
 
Infective Endocarditis in Children
Infective Endocarditis in ChildrenInfective Endocarditis in Children
Infective Endocarditis in Children
 
acute rheumatic fever
acute rheumatic feveracute rheumatic fever
acute rheumatic fever
 
Infective Endocarditis
Infective EndocarditisInfective Endocarditis
Infective Endocarditis
 
Rheumatic Heart disease
Rheumatic Heart diseaseRheumatic Heart disease
Rheumatic Heart disease
 

Semelhante a Acute rheumatic fever-definition,pathophysiology,clinical presentation and management.

Acute rheumatic fever
Acute  rheumatic  fever Acute  rheumatic  fever
Acute rheumatic fever
jj Liyanage
 
Acute rheumatic fever
Acute  rheumatic feverAcute  rheumatic fever
Acute rheumatic fever
drsharan77
 
management of acute rheumatic fever
management of acute rheumatic fevermanagement of acute rheumatic fever
management of acute rheumatic fever
Basem Enany
 
RHEUMATIC DISEASE OF CHILDHOOD(BINGHAM UNIVERSITY)_074613.pptx
RHEUMATIC DISEASE OF CHILDHOOD(BINGHAM UNIVERSITY)_074613.pptxRHEUMATIC DISEASE OF CHILDHOOD(BINGHAM UNIVERSITY)_074613.pptx
RHEUMATIC DISEASE OF CHILDHOOD(BINGHAM UNIVERSITY)_074613.pptx
EmmanuelElijah8
 
SLE by DRMAGDI SASI 2016
SLE by DRMAGDI SASI 2016SLE by DRMAGDI SASI 2016
SLE by DRMAGDI SASI 2016
cardilogy
 
Rheumatic fever
Rheumatic feverRheumatic fever
Rheumatic fever
KemUnited
 

Semelhante a Acute rheumatic fever-definition,pathophysiology,clinical presentation and management. (20)

Acute rheumatic fever
Acute  rheumatic  fever Acute  rheumatic  fever
Acute rheumatic fever
 
Acute rheumatic fever
Acute rheumatic fever Acute rheumatic fever
Acute rheumatic fever
 
Acute rheumatic fever
Acute  rheumatic feverAcute  rheumatic fever
Acute rheumatic fever
 
Rheumatic fever
Rheumatic feverRheumatic fever
Rheumatic fever
 
ARF DEV (1).pptx
ARF DEV (1).pptxARF DEV (1).pptx
ARF DEV (1).pptx
 
management of acute rheumatic fever
management of acute rheumatic fevermanagement of acute rheumatic fever
management of acute rheumatic fever
 
RHEUMATIC DISEASE OF CHILDHOOD(BINGHAM UNIVERSITY)_074613.pptx
RHEUMATIC DISEASE OF CHILDHOOD(BINGHAM UNIVERSITY)_074613.pptxRHEUMATIC DISEASE OF CHILDHOOD(BINGHAM UNIVERSITY)_074613.pptx
RHEUMATIC DISEASE OF CHILDHOOD(BINGHAM UNIVERSITY)_074613.pptx
 
Rheumatoid arthritis
Rheumatoid arthritisRheumatoid arthritis
Rheumatoid arthritis
 
Acute rheumatic fever
Acute rheumatic feverAcute rheumatic fever
Acute rheumatic fever
 
Rheumatic fever
Rheumatic feverRheumatic fever
Rheumatic fever
 
SLE by DRMAGDI SASI 2016
SLE by DRMAGDI SASI 2016SLE by DRMAGDI SASI 2016
SLE by DRMAGDI SASI 2016
 
Rheumatic fever and Rheumatic heart disease
Rheumatic fever and Rheumatic heart diseaseRheumatic fever and Rheumatic heart disease
Rheumatic fever and Rheumatic heart disease
 
Sarcoidosis
SarcoidosisSarcoidosis
Sarcoidosis
 
10.Rheumatic Fever.ppt
10.Rheumatic Fever.ppt10.Rheumatic Fever.ppt
10.Rheumatic Fever.ppt
 
Rheumatic fever
Rheumatic feverRheumatic fever
Rheumatic fever
 
3 arf (2)
3 arf (2)3 arf (2)
3 arf (2)
 
Lupus overview for journalist
Lupus overview for journalistLupus overview for journalist
Lupus overview for journalist
 
rheumatic_fever.ppt
rheumatic_fever.pptrheumatic_fever.ppt
rheumatic_fever.ppt
 
seminar on ARF.pptx
seminar on ARF.pptxseminar on ARF.pptx
seminar on ARF.pptx
 
ACUTE RHEUMATIC FEVER PPT.pptx
ACUTE RHEUMATIC FEVER PPT.pptxACUTE RHEUMATIC FEVER PPT.pptx
ACUTE RHEUMATIC FEVER PPT.pptx
 

Último

Call Girls in Gagan Vihar (delhi) call me [🔝 9953056974 🔝] escort service 24X7
Call Girls in Gagan Vihar (delhi) call me [🔝  9953056974 🔝] escort service 24X7Call Girls in Gagan Vihar (delhi) call me [🔝  9953056974 🔝] escort service 24X7
Call Girls in Gagan Vihar (delhi) call me [🔝 9953056974 🔝] escort service 24X7
9953056974 Low Rate Call Girls In Saket, Delhi NCR
 

Último (20)

Model Call Girls In Chennai WhatsApp Booking 7427069034 call girl service 24 ...
Model Call Girls In Chennai WhatsApp Booking 7427069034 call girl service 24 ...Model Call Girls In Chennai WhatsApp Booking 7427069034 call girl service 24 ...
Model Call Girls In Chennai WhatsApp Booking 7427069034 call girl service 24 ...
 
Call Girls Kurnool Just Call 8250077686 Top Class Call Girl Service Available
Call Girls Kurnool Just Call 8250077686 Top Class Call Girl Service AvailableCall Girls Kurnool Just Call 8250077686 Top Class Call Girl Service Available
Call Girls Kurnool Just Call 8250077686 Top Class Call Girl Service Available
 
Russian Call Girls Service Jaipur {8445551418} ❤️PALLAVI VIP Jaipur Call Gir...
Russian Call Girls Service  Jaipur {8445551418} ❤️PALLAVI VIP Jaipur Call Gir...Russian Call Girls Service  Jaipur {8445551418} ❤️PALLAVI VIP Jaipur Call Gir...
Russian Call Girls Service Jaipur {8445551418} ❤️PALLAVI VIP Jaipur Call Gir...
 
Call Girls Gwalior Just Call 8617370543 Top Class Call Girl Service Available
Call Girls Gwalior Just Call 8617370543 Top Class Call Girl Service AvailableCall Girls Gwalior Just Call 8617370543 Top Class Call Girl Service Available
Call Girls Gwalior Just Call 8617370543 Top Class Call Girl Service Available
 
Call Girls in Gagan Vihar (delhi) call me [🔝 9953056974 🔝] escort service 24X7
Call Girls in Gagan Vihar (delhi) call me [🔝  9953056974 🔝] escort service 24X7Call Girls in Gagan Vihar (delhi) call me [🔝  9953056974 🔝] escort service 24X7
Call Girls in Gagan Vihar (delhi) call me [🔝 9953056974 🔝] escort service 24X7
 
Call Girls Kakinada Just Call 9907093804 Top Class Call Girl Service Available
Call Girls Kakinada Just Call 9907093804 Top Class Call Girl Service AvailableCall Girls Kakinada Just Call 9907093804 Top Class Call Girl Service Available
Call Girls Kakinada Just Call 9907093804 Top Class Call Girl Service Available
 
Mumbai ] (Call Girls) in Mumbai 10k @ I'm VIP Independent Escorts Girls 98333...
Mumbai ] (Call Girls) in Mumbai 10k @ I'm VIP Independent Escorts Girls 98333...Mumbai ] (Call Girls) in Mumbai 10k @ I'm VIP Independent Escorts Girls 98333...
Mumbai ] (Call Girls) in Mumbai 10k @ I'm VIP Independent Escorts Girls 98333...
 
Call Girls Service Jaipur {8445551418} ❤️VVIP BHAWNA Call Girl in Jaipur Raja...
Call Girls Service Jaipur {8445551418} ❤️VVIP BHAWNA Call Girl in Jaipur Raja...Call Girls Service Jaipur {8445551418} ❤️VVIP BHAWNA Call Girl in Jaipur Raja...
Call Girls Service Jaipur {8445551418} ❤️VVIP BHAWNA Call Girl in Jaipur Raja...
 
💕SONAM KUMAR💕Premium Call Girls Jaipur ↘️9257276172 ↙️One Night Stand With Lo...
💕SONAM KUMAR💕Premium Call Girls Jaipur ↘️9257276172 ↙️One Night Stand With Lo...💕SONAM KUMAR💕Premium Call Girls Jaipur ↘️9257276172 ↙️One Night Stand With Lo...
💕SONAM KUMAR💕Premium Call Girls Jaipur ↘️9257276172 ↙️One Night Stand With Lo...
 
Top Rated Hyderabad Call Girls Erragadda ⟟ 9332606886 ⟟ Call Me For Genuine ...
Top Rated  Hyderabad Call Girls Erragadda ⟟ 9332606886 ⟟ Call Me For Genuine ...Top Rated  Hyderabad Call Girls Erragadda ⟟ 9332606886 ⟟ Call Me For Genuine ...
Top Rated Hyderabad Call Girls Erragadda ⟟ 9332606886 ⟟ Call Me For Genuine ...
 
Premium Call Girls In Jaipur {8445551418} ❤️VVIP SEEMA Call Girl in Jaipur Ra...
Premium Call Girls In Jaipur {8445551418} ❤️VVIP SEEMA Call Girl in Jaipur Ra...Premium Call Girls In Jaipur {8445551418} ❤️VVIP SEEMA Call Girl in Jaipur Ra...
Premium Call Girls In Jaipur {8445551418} ❤️VVIP SEEMA Call Girl in Jaipur Ra...
 
Jogeshwari ! Call Girls Service Mumbai - 450+ Call Girl Cash Payment 90042684...
Jogeshwari ! Call Girls Service Mumbai - 450+ Call Girl Cash Payment 90042684...Jogeshwari ! Call Girls Service Mumbai - 450+ Call Girl Cash Payment 90042684...
Jogeshwari ! Call Girls Service Mumbai - 450+ Call Girl Cash Payment 90042684...
 
The Most Attractive Hyderabad Call Girls Kothapet 𖠋 9332606886 𖠋 Will You Mis...
The Most Attractive Hyderabad Call Girls Kothapet 𖠋 9332606886 𖠋 Will You Mis...The Most Attractive Hyderabad Call Girls Kothapet 𖠋 9332606886 𖠋 Will You Mis...
The Most Attractive Hyderabad Call Girls Kothapet 𖠋 9332606886 𖠋 Will You Mis...
 
Call Girls Vasai Virar Just Call 9630942363 Top Class Call Girl Service Avail...
Call Girls Vasai Virar Just Call 9630942363 Top Class Call Girl Service Avail...Call Girls Vasai Virar Just Call 9630942363 Top Class Call Girl Service Avail...
Call Girls Vasai Virar Just Call 9630942363 Top Class Call Girl Service Avail...
 
Top Rated Bangalore Call Girls Mg Road ⟟ 9332606886 ⟟ Call Me For Genuine S...
Top Rated Bangalore Call Girls Mg Road ⟟   9332606886 ⟟ Call Me For Genuine S...Top Rated Bangalore Call Girls Mg Road ⟟   9332606886 ⟟ Call Me For Genuine S...
Top Rated Bangalore Call Girls Mg Road ⟟ 9332606886 ⟟ Call Me For Genuine S...
 
VIP Service Call Girls Sindhi Colony 📳 7877925207 For 18+ VIP Call Girl At Th...
VIP Service Call Girls Sindhi Colony 📳 7877925207 For 18+ VIP Call Girl At Th...VIP Service Call Girls Sindhi Colony 📳 7877925207 For 18+ VIP Call Girl At Th...
VIP Service Call Girls Sindhi Colony 📳 7877925207 For 18+ VIP Call Girl At Th...
 
Call Girls Service Jaipur {9521753030} ❤️VVIP RIDDHI Call Girl in Jaipur Raja...
Call Girls Service Jaipur {9521753030} ❤️VVIP RIDDHI Call Girl in Jaipur Raja...Call Girls Service Jaipur {9521753030} ❤️VVIP RIDDHI Call Girl in Jaipur Raja...
Call Girls Service Jaipur {9521753030} ❤️VVIP RIDDHI Call Girl in Jaipur Raja...
 
Pondicherry Call Girls Book Now 9630942363 Top Class Pondicherry Escort Servi...
Pondicherry Call Girls Book Now 9630942363 Top Class Pondicherry Escort Servi...Pondicherry Call Girls Book Now 9630942363 Top Class Pondicherry Escort Servi...
Pondicherry Call Girls Book Now 9630942363 Top Class Pondicherry Escort Servi...
 
Call Girls Raipur Just Call 9630942363 Top Class Call Girl Service Available
Call Girls Raipur Just Call 9630942363 Top Class Call Girl Service AvailableCall Girls Raipur Just Call 9630942363 Top Class Call Girl Service Available
Call Girls Raipur Just Call 9630942363 Top Class Call Girl Service Available
 
Top Rated Bangalore Call Girls Richmond Circle ⟟ 9332606886 ⟟ Call Me For Ge...
Top Rated Bangalore Call Girls Richmond Circle ⟟  9332606886 ⟟ Call Me For Ge...Top Rated Bangalore Call Girls Richmond Circle ⟟  9332606886 ⟟ Call Me For Ge...
Top Rated Bangalore Call Girls Richmond Circle ⟟ 9332606886 ⟟ Call Me For Ge...
 

Acute rheumatic fever-definition,pathophysiology,clinical presentation and management.

  • 1.
  • 2.  INTRODUCTION  EPIDEMIOLOGY  PATHOGENESIS  PATHOLOGY  CLINICAL FEATURES  LABORATORY FINDINGS  DIAGNOSIS  TREATMENT  PREVENTION  REFERENCES
  • 3. Acute rheumatic fever (ARF) is a multisystem disease resulting from an autoimmune reaction to infection with group A streptococci.  ARF and RHD are diseases of poverty.  It is a postsuppurative streptococcal pharyngitis cascade, leading variably to arthritis , chorea , dermal manifestations and most importantly, carditis.
  • 4.  The incidence of RF has declined dramatically in industrialized nations , but remains common in developing nations. Accounts for less than 1% of cardiac manifestations in industrialized countries. RHD is the most common cause of heart disease in children in developing countries and is a major cause of mortality and morbidity in adults as well.  Estimated in 2005 that approximately 15.6 million people had RF or RHD.
  • 5.  Age group:5 to 14 years.  Initial episodes become less common in older adolescents and young adults and are rare in persons aged >30 years.  Recurrent episodes of ARF remain relatively common in adolescents and young adults.  No clear gender , race predisposition  But post-pubertal chorea and the development of mitral stenosis is more common in females.
  • 6.  Classic triad of agent ,host and environmental all play a major role. AGENT FACTORS:  infection of the upper respiratory tract with group A beta hemolytic streptococci.  more than 100 subtypes defined by M protein surface molecules.  strains that cause rheumatic fever are -M types 1,3,5,6,18 and 24.
  • 7. Diagrammatic structure of the group A beta hemolytic streptococcus Capsule Cell wall Protein antigens Group carbohydrate Peptidoglycan Cyto.membrane Cytoplasm
  • 8. HOST FACTORS: Susceptibility to ARF is an inherited characteristic.  HLA class II alleles.  High levels of circulating mannose-binding lectin  Polymorphisms of transforming growth factor β1 gene and immunoglobulin genes.  High-level expression of a particular alloantigen present on B cells, D8-17, has been found in patients with a history of ARF in many populations.
  • 9.  Prior history of rheumatic fever is also an important risk factor. ENVIRONMENTAL FACTORS:  overcrowded housing,  poor personal and community hygiene,  poor access to medical services.
  • 10. THE IMMUNE RESPONSE:  An autoimmune reaction results, which leads to damage to human tissues.  Cross-reactivity between epitopes on the organism and the host.  Epitopes present in the cell wall, cell membrane, and the A, B, and C repeat regions of the streptococcal M protein are immunologically similar to molecules in human:  myosin, tropomyosin(myocardium),  keratin (skin)  actin,
  • 11.  laminin(valves),  vimentin(synovia),  N-acetylglucosamine.  lysogangliosides(subthalamic and caduate nuclei in the brain).  This molecular mimicry is the basis for the autoimmune response that leads to ARF.
  • 12.  Laminin, α-helical coiled protein like myosin and M protein, found in cardiac endothelium recognized by anti-myosin, anti-M protein T cells.  Antibodies to cardiac valve tissue cross-react with the N-acetylglucosamine of group A streptococcal carbohydrate.  These antibodies may be responsible for valvular damage.
  • 13. Pathogenetic pathway for acute rheumatic fever and rheumatic heart disease.
  • 14.  Verrucous vegetations on the valve leaflets along the lines of closure, with extensive inflammation and edema.
  • 15. EXUDATIVE PHASE:  first few weeks after the onset of RF  fibrinoid degeneration of collagen  inflammation in left ventricular endocardium. PROLIFERATIVE PHASE:  from 1 to 6 months after the onset of RF.  ASCHOFF BODIES ,granulomatous lesions pathognomonic for rheumatic carditis .  found in valve tissue as well as endocardium, myocardium and pericardium.
  • 16. Microscopic appearance of Aschoff body in a patient with acute rheumatic carditis.
  • 17. Rheumatic arthritis is manifest by  edema, lymphocytic and polymorphonuclear infiltration,  fibrinoid lesions that resolve. In patients with chorea , inflammatory changes have been noted in the cerebral cortex ,cerebellum and basal ganglia.
  • 18.  Latent period of ~3 weeks (1–5 weeks) between the precipitating group A streptococcal infection and the appearance of the clinical features of ARF. Exceptions are chorea and indolent carditis,which may follow prolonged latent periods lasting up to 6 months. Clinical manifestations:  Fever  Polyarthritis  Carditis
  • 19.  Sydenham’s chorea  Erythema marginatum.  Subcutaneous nodules. Most common is fever ,polyarthritis followed by carditis.
  • 20. CARDITIS: Occurs in 50-60% of patients with ARF.  The endocardium, pericardium, or myocardium may be affected.  Valvular damage is the hallmark of rheumatic carditis.  almost always affects the mitral valve causing MR.  sometimes together with aortic valve.  isolated aortic valve involvement with AR is rare.  acute and chronic myocardial dysfunction.  acute ,although not chronic pericardial disease.
  • 21.  neither pericarditis nor myocarditis can be expected to occur in the absence of valvulitis.  Rheumatic tricuspid disease is uncommon ,and pulmonic valve disease is rare.  Severe heart failure in acute RF is secondary to altered myocardial mechanics caused by MR rather than secondary to myocarditis. • Severity of left ventricular dysfunction appears to correlate with the extent of valvulitis rather than with any myocardial injury.
  • 22. Over ensuing years, usually as a result of recurrent episodes, leaflet thickening, scarring, calcification, and valvular stenosis may develop.
  • 23. Pericarditis most commonly causes a friction rub or a small effusion on echocardiography and may occasionally cause pleuritic central chest pain. 
  • 24. Myocardial inflammation may affect electrical conduction pathways ,leading to P-R interval prolongation (first-degree AV block or rarely higher- level block) which resolves over a few days to weeks.
  • 25.  JOINT INVOLVEMENT:  Polyarthrits is most common manifestation of RF , occuring in 60 to 75% of patients. To qualify as a major manifestation, joint involvement must be arthritic, i.e., objective evidence of inflammation ,with hot, swollen, red and/or tender joints and involvement of more than one joint (i.e., polyarthritis).  typically migratory ,moving from one joint to another over a period of hours.  almost always affects the large joints— the knees, ankles, hips, and elbows  asymmetric.
  • 26.  Arthralgia without objective joint inflammation - minor criteria.  Inflammation in individual joints lasts 1 to 2 weeks.  Polyarthritis as a whole resolves in a month or less.  Chronic sequelae and disability do not occur ,with the rare exception of Jaccoud arthropathy.
  • 27. • Arthritic phase frequently overlaps with the onset of carditis and the two manifestations appear to be inversely proportional.  Joint manifestations are highly responsive to salicylates and other nonsteroidal anti-inflammatory drugs.
  • 28. CHOREA  Sydenham’s chorea occurs after a prolonged latent period of upto 6 months.  Reported incidence has a wide range between 5 and 35%.  Found mainly in females.  Affects particularly the head and upper limb.  Manifests as involuntary , irregular movements, fibrillatory muscle movements of tongue.
  • 29.  Characteristic spooning with exterernal rotation of the hands.  Abolition with sleep.  There is a substantial risk of subsequent RHD in these patients.  Eventually resolves completely, usually within 6 weeks.  Other psychological and neurological manifestations of RF:  Short term and long term emotional liability.  Obsessive –compulsive disorder.
  • 31. SKIN MANIFESTATIONS: Occur in <5% of the cases. Erythema marginatum:  Classic rash of ARF  Begins as pink macules that clear centrally, leaving a serpiginous, spreading edge.  The rash is evanescent, appearing and disappearing before the examiner’s eyes.  It occurs usually on the trunk, sometimes on the limbs, but almost never on the face.  Typically occur in conjunction with carditis.
  • 32.  May last for months or years.  Not specific for RF and occurs with sepsis and drug reaction.
  • 33. Subcutaneous nodules:  Painless, small , mobile lumps beneath the skin overlying bony prominences, of the hands, feet, elbows, occiput, and occasionally the vertebrae.  Typically occur in patients with moderate to severe rheumatic carditis.  They are a delayed manifestation, appearing 2–3 weeks after the onset of disease, last for just a few days up to 3 weeks.  Not diagnostic of RF and can be seen with other autoimmune disorders.
  • 35. OTHER FEATURES:  Fever occurs in most cases of ARF.  Although high-grade fever (≥39°C) is the rule, lower grade temperature elevations are not uncommon.
  • 37. Connective tissue disease Rheumatoid arthritis Systemic lupus Systemic vasculitis  Miscellaneous : Gout, Leukemia, lymphoma , Sarcoidosis, Cancer , Familial Mediterranean fever , Henoch-Schönlein purpura , Mucocutaneous disorders , “Growth pains” in children, Serum sickness .
  • 38. Carditis  Murmur Physiological murmur Mitral valve prolapse Bicuspid aortic valve Anemia Straight back syndrome  Congenital heart disease Ventricular septal defect Subvalvular aortic stenosis Primum atrial septal defect  Viral myocarditis  Endocarditis  Pericarditis
  • 39. Chorea  Familial chorea—Huntington  Hormone-induced Oral contraceptives, pregnancy  Drug-induced Anticonvulsants , antidepressant, metoclopramide.  Connective tissue Systemic lupus, Periarteritis  Lyme disease , Wilson disease , Atypical seizures , Hyperthyroidism, Hypoparathyroidism , Tourette syndrome, PANDAs.
  • 40. Elevated ESR and CRP.  Mild elevation of the peripheral leucocyte count.  Evidence of a Preceding Group A Streptococcal Infection:  Positive throat swab culture  Rapid streptococcal antigen test-generally specific but sensitivity is low  Rising streptococcal antibody titres which includes: a. antistreptolysin O. b. anti-deoxyribonuclease B.
  • 41. c. antihyaluronidase d. streptozyme. Antibody titre testing is more specific although they are affected by non-GABHS infections. Time course of antibody level increase:  within 1 month of onset of streptococcal pharyngitis.  plateaus for 3 t0 6 months, followed by a decline.  levels elevated from the patient’s baseline typically last 1 year or less.
  • 42.  Electrocardiographic findings:  Sinus arrythmia  Tachycardia  Conduction disturbances-first degree AV block.  Prolongation of QT interval.  Rare episodes of torsades de pointes and sudden death.
  • 43.  Echocardiography:  Miral insufficiency is the most common finding associated subsequently with restricted leaflet motion  Occasionally aortic insufficiency has been diagnosed in a minority of patients. Echocardiography is useful :  For confirming the findings on auscultation.  Excluding non-rheumatic causes(physiological murmurs or congenital heart disease).  Sequential follow up of valvular insufficiency,
  • 44. chamber size ,pulmonary hypertension, valve thickening and left ventricular systolic function.
  • 45.  Associated post-streptococcal syndromes:  Post streptococcal reactive arthritis: (1) small-joint involvement that is often symmetric. (2) a short latent period following streptococcal infection (usually <1 week). (3) occasional causation by non-group A β-hemolytic streptococcal infection. (4) slower responsiveness to salicylates. (5) the absence of other features of ARF, particularly carditis.
  • 46.  Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infection (PANDAS) : Consists of a range of tic disorders and obsessive- compulsive symptoms associated with group A streptococcal infections.  People with PANDAS are said not to be at risk of carditis, unlike patients with Sydenham’s chorea.  The diagnoses of PANDAS and PSRA should rarely be made in populations with a high incidence of ARF
  • 47.  Based on the WHO modification of the 1992 Revised Jones criteria. Major manifestations:  Carditis  Polyarthritis  Chorea  Erythema marginatum  Subcutaneous nodules
  • 48.  Minor manifestations:  Clinical: fever, polyarthralgia  Laboratory: elevated erythrocyte sedimentation rate or leukocyte count  Electrocardiogram: prolonged P-R interval Supporting evidence of a preceding streptococcal infection within the last 45 days  Elevated or rising anti-streptolysin O or other streptococcal antibody, or  A positive throat culture, or  Rapid antigen test for group A streptococcus, or  Recent scarlet fever
  • 49. Primary episode of rheumatic fever:  Two major or one major and two minor manifestations plus evidence of preceding group A streptococcal infection. Recurrent attack of rheumatic fever in a patient without established rheumatic heart disease:  Two major or one major and two minor manifestations plus evidence of preceding group A streptococcal infection
  • 50. Recurrent attack of rheumatic fever in a patient with established rheumatic heart disease.  Two minor manifestations plus evidence of preceding group A streptococcal infection. Rheumatic chorea and Insidious onset rheumatic carditis:  Other major manifestations or evidence of group A streptococcal infection not required.
  • 51. Chronic valve lesions of rheumatic heart disease (patients presenting for the first time with pure mitral stenosis or mixed mitral valve disease and/or aortic valve disease)  Do not require any other criteria to be diagnosed as having rheumatic heart disease.
  • 52. ANTIBIOTICS:  All patients with ARF should receive antibiotics sufficient to treat the precipitating group A streptococcal infection.  Penicillin is the drug of choice  can be given orally , 500 mg twice daily for 10 days or  as a single dose of 1.2 million units IM benzathine penicillin G.  Erythromycin, 250 mg bid, may be used for patients with penicillin allergy.  Followed by long term secondary prophylaxis.
  • 53.  SALICYLATES AND NSAIDS Used for the treatment of arthritis, arthralgia, and fever, once the diagnosis is confirmed.  Aspirin is the drug of choice.  Initial dose of 80–100 mg/kg per day in children (4–8 g/d in adults) in 4–5 divided doses for the first few days up to 2 weeks.  When the acute symptoms are substantially resolved, the dose can be reduced to 60–70 mg/kg per day for a further 2–4 weeks.
  • 54.  GLUCOCORTICOIDS:  Can be used in cases of severe carditis (causing heart failure) may reduce the acute inflammation and result in more rapid resolution of failure.  Prednisolone:1–2 mg/kg per day (maximum, 80 mg) Intravenous methylprednisolone may be used in very severe carditis.  Often required for a few days or up to a maximum of 3 weeks.
  • 55.  MANAGEMENT OF HEART FAILURE  BED REST: Recommended only for arthritis and arthralgia and for patients with heart failure.  MANAGEMENT OF CHOREA  Milder cases can usually be managed by providing a calm environment.  In severe chorea, carbamazepine or sodium valproate are preferred to haloperidol.
  • 56.  Response may not be seen for 1–2 weeks.  Medication should be continued for 1–2 weeks after symptoms subside. INTRAVENOUS IMMUNOGLOBULIN(IVIG):  Studies have suggested that IVIg may lead to more rapid resolution of chorea.  But has shown no benefit on the short- or long-term outcome of carditis in ARF without chorea.  IVIg is not recommended except in cases of severe chorea refractory to other treatments.
  • 57.  PRIMARY PREVENTION: I. Elimination of the major risk factors for streptococcal infection, particularly overcrowded housing and inadequate hygiene infrastructure. II. Primary prophylaxis:  Timely and complete treatment of group A streptococcal sore throat with antibiotics.  If commenced within 9 days of sore throat onset, a course of 10 days of penicillin V (500 mg bid PO in adults) or
  • 58.  A single IM injection of 1.2 million units of benzathine penicillin G should be administered. SECONDARY PREVENTION:  The mainstay of controlling ARF and RHD.  Patients with ARF should receive long term penicillin prophylaxis to prevent recurrences.  The best antibiotic for secondary prophylaxis is benzathine penicillin G: 1.2 million units, or 600,000 units if <30 kg delivered every 4 weeks or more frequently to persons considered to be at particularly high risk.
  • 59.  Oral penicillin V (250 mg) can be given twice-daily instead.  less effective than benzathine penicillin G. Penicillin allergic patients can receive erythromycin (250 mg) twice daily.
  • 60.
  • 61. Harrison’s Principles of Internal Medicine,17th edt.  Braunwald’s diseases of the heart,8th edt.  Oxford text book of medicine -4th edt.  Nelson’s text book of Pediatrics.  Robbins and Cotran ,Pathological Basis of Diseases,8th edt.  Cecil text book of Medicine,22nd edt.